Spinal Cord Syndrome Due to Extramedullary Epithelioid Hemangioendothelioma of the Thoracic Spine: A Case Report and Literature Review.

We report a 48-year-old male patient with spinal epithelioid hemangioendothelioma in T3 and T4 who began with symptoms of paresthesia in the lower limbs and distal weakness of the right lower limb, back pain, increased limitation in walking, urinary incontinence, and constipation. A safe maximum resection was performed, finding residual disease during the PET/CT scan, so it was decided to treat with radiotherapy, and there was a good response to this treatment. A literature review of epithelioid hemangioendothelioma of the thoracic spine was done which showed a mean age of presentation of 41 years and a male-female ratio of 1:0.53. The main symptom was pain, which was present in 100% of the patients, and wide surgery was performed in 56.8% of the patients, intralesional surgery in 31.8%, and biopsy in 11.4%. A total of 46.6% of patients received radiation therapy, and only 6.6% received chemotherapy. The patients had an average follow-up of 38 months. We recommend that extension studies such as PET/CT scans be performed after surgical resection. This can serve as a follow-up with hemangioendothelioma epithelioma patients about metastatic disease or residual disease that will guide us in giving adjuvant treatments, such as radiotherapy or chemotherapy, for better control of the disease.

A Technical Note on Alternating Laminectomies Plus Folding Cystectomy: A Novel Technique for Spinal Arachnoid Cyst.

We report the case of a 33-year-old male patient with no past medical history presenting to our tertiary referral center with progressive (two years) deficit of lower limb motor impairment (2/5 Medical Research Council [MRC] scale) and sensory impairment. T2- and T1-weighted MRI images clarified the nature of the cyst from T3 to T8. In our case, surgical management was warranted to relieve tension over the spinal cord, thus improving symptoms. Two multilevel laminectomies were performed, one centered on the proximal pole and the other on the distal pole; subsequently, the epidural cyst was gradually folded until it was totally extracted without complications. In the present study, we discuss a technique of extended spinal compressive arachnoid cyst. To the best of our knowledge, this technique has not been previously described in the existing body of literature. Here, we present a case of a successful procedure that seems both efficient and safe. Further study will be required to confirm its safety and efficacy.

Mature Spinal Teratoma: A Case Report.

We presented an unusual case of a teratoma in a 76-year-old female who began four years ago with paresthesias and hypoesthesias in the sacral and gluteal regions. She denied weakness or gait instability. The magnetic resonance imaging showed an intradural lesion within the cauda equina at levels L2-L3. We decided to perform a posterior midline approach to the lumbar region to expose L2-L3 levels. After doing the L2-L3 laminectomy and the durotomy, we found a solid lesion surrounded by nerve roots with heterogeneous content. Through the meticulous separation of the nerve roots surrounding the lesion, we punctioned it, observing the exit of melanocytic material. Histopathological findings showed germinal neoplasia without immature neuroepithelium or malignant component; therefore, the diagnosis of mature teratoma was made. The patient was discharged without any aggregate neurological deficit. At the six-month follow-up visit, the patient continued with paresthesia in the gluteal region without motor weakness and reported minimal gait improvement.

Calcified Pilocytic Astrocytomas and Calcifying Pseudoneoplasms of the Neuraxis: A Diagnostic Challenge.

Pilocytic astrocytoma (PA), recognized as the most prevalent central nervous system (CNS) tumor, has long been associated with calcifications, a characteristic often attributed to benign or indolent growth patterns. In this study, we explored the calcified attributes in these tumors that beckon a deeper understanding. This is a retrospective study, on a set of seven cases, with a histopathological diagnosis of pilocytic astrocytoma with calcifications and psammoma bodies (PB). Despite an encouraging overall survival outcome, the recurrence in four cases cast some doubt on the conventional classification. The histological study of these cases revealed a spectrum of calcifications, varying in size and morphology, all of which exhibited positive reactivity to glial fibrillary acidic protein (GFAP), osteoconduction, and osteopontin. Notably, the immunohistochemistry showed hyaline bodies displaying an atypical immune profile, strikingly negative for vimentin and GFAP, and a robust positivity for epidermal growth factor receptors (EGFR), tumor necrosis factor-alpha (TNF-α), and interleukin 1 beta (IL-1ß). These results stimulated speculation that the identity of these calcified tumors may have extended and potentially embraced the realm of calcifying pseudoneoplasms of the neuraxis (CAPNON), underscored by intense pilot gliosis. This study transcends mere anatomical exploration; it delves into the intricacies of calcified tumors, casting a spotlight on the dynamic interplay between PA and CAPNON. As we traverse the frontiers of neuro-oncology, these findings pave the way for innovative avenues in the diagnostics and therapeutics of these tumors.

Mature Teratoma at the Lumbar Spinal Cord: A Case Report and Literature Review.

Mature spinal teratoma is a rare type of germ cell tumor that arises from any of the three germ cell layers (ectoderm, mesoderm, and endoderm) and consists of differentiated tissues and structures that reflect the cellular organization and morphology of normal adult tissues. It has the ability to grow independently and cause compressive symptoms when found in this rare location. In this article, we present the case of a 29-year-old male patient with the onset of neurological symptoms beginning with pelvic limb paresthesias and progressing to back pain. Magnetic resonance imaging (MRI) revealed a tumor at L1-L4, which was resected by laminotomy, and histopathology revealed a mature intradural teratoma. Fortunately, this histologic type had a good prognosis for our patient, who had a significant clinical improvement. A systematic review was performed using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) methodology with PubMed and Google Scholar to find similar case reports and to summarize the main features of this disease, which contributes to the understanding of its diagnostic presentation, treatment, and prognosis, improving clinical practice in the management of similar cases. The rarity of this condition, together with its wide clinical heterogeneity and prognosis, underscores the importance of a thorough evaluation of cases of intramedullary lesions, where the consideration of uncommon diseases in the differential diagnosis should be highlighted.

Surgical outcomes in patients with acromegaly: Microscopic vs. endoscopic transsphenoidal surgery.

Transsphenoidal resection of growth hormone-secreting pituitary neuroendocrine tumors remains the first-line treatment for acromegaly. This can be performed through microsurgery or endoscopic surgery. For the past decades, endoscopic surgery has become the preferred technique in an increasing number of centers worldwide. However, whether it offers superior clinical outcomes has yet to be determined. In this paper, we performed a narrative review of the literature comparing both techniques in the treatment of acromegaly. We critically assessed available comparative studies from an objective perspective to determine their suitability for defining superiority of either technique. Available evidence displays substantial methodological variations and reports conflicting findings. Although endoscopic surgery provides a wider exposure and enhanced visibility of the surgical field, this does not consistently translate into better clinical outcomes, as most tumors are equally accessible through both techniques. Postoperative outcomes such as remission and complication rates are similar between both techniques. The management of acromegaly should be performed by experienced pituitary neurosurgeons, regardless of the approach. The involvement of a multidisciplinary team in a dedicated pituitary center is critical to ensure optimal outcomes.

Spinal Ependymomas: An Updated WHO Classification and a Narrative Review.

Ependymomas are neuroepithelial tumors that develop from ependymal cells found in the brain parenchyma and can spread to any part of the spinal cord. Three to six percent of all malignancies affecting the central nervous system (CNS) are ependymomas. Even the most talented surgeons are challenged by spinal cord ependymomas; as a result, research into this clinical phenomenon should continue. Since 1979, the World Health Organization (WHO) has published a classification and grading system for CNS malignancies to ensure consistent diagnostic standards worldwide. The WHO prepared an update on these tumors, paying particular attention to molecular techniques to categorize the therapeutic management of each patient with greater accuracy and clarity. We thoroughly reviewed the literature on the epidemiology, etiology, diagnosis, and treatment of spinal ependymomas since there has not been a recent review of these tumors. This included modifications to the 2021 WHO Classification of Tumors of the Central Nervous System.

Relative cerebral blood volume as response predictor in the treatment of recurrent glioblastoma with anti-angiogenic therapy.

BACKGROUND: Glioblastoma is one of the most common brain tumors in adult populations, usually carrying a poor prognosis. While several studies have researched the impact of anti-angiogenic therapies, especially anti-VEFG treatments in glioblastoma, few have attempted to assess its progress using imaging studies. PURPOSE: We attempted to analyze whether relative cerebral blood volume (rCBV) from dynamic susceptibility-weighted contrast-enhanced MRI (DSC-MRI) could predict response in patients with glioblastoma undergoing Bevacizumab (BVZ) treatment. METHODS: We performed a retrospective study evaluating patients with recurrent glioblastoma receiving anti-angiogenic therapy with BVZ between 2012 and 2017 in our institution. Patients were scheduled for routine MRIs at baseline and first-month follow-up visits. Studies were processed for DSC-MRI, cT1, and FLAIR images, from which relative cerebral blood volume measurements were obtained. We assessed patient response using the Response Assessment in Neuro-Oncology (RANO) working group criteria and overall survival. RESULTS: 40 patients were included in the study and were classified as Bevacizumab responders and non-responders. The average rCBV before treatment was 4.5 for both groups, and average rCBV was 2.5 for responders and 5.4 for non-responders. ROC curve set a cutoff point of 3.7 for rCBV predictive of response to BVZ. Cox Multivariate analysis only showed rCBV as a predictive factor of OS. CONCLUSION: A statistically significant difference was found in rCBV between patients who responded and those who did not respond to BVZ treatment. rCBV may be a low-cost and effective marker to assess response to Bevacizumab treatment in GBM.

Foix-Alajouanine Syndrome: A Case Report.

Foix-Alajouanine syndrome is a rare form of presentation of an arteriovenous malformation of the spinal cord that causes myelopathy in the thoracic and lumbar medullary segments. We present the case of a 46-year-old female who suffered from weakness in the lower limbs with sensation loss, low back pain, urinary incontinence, and constipation. The magnetic resonance image T2 sequence of the thoracic spine from T6 to T11 revealed abnormally hypointense signals in the posterior epidural region caused by larger arteries. A spinal digital subtraction angiography was useful to diagnose a right perimedullary fistula with venous drainage, which was satisfactorily embolized. The key to suspecting this diagnosis is the presence of dilated vessels in the posterior epidural space, which are evident in T2 and short tau inversion recovery (STIR)-weighted sequences. Physicians often misdiagnose Foix-Alajouanine syndrome, resulting in potential delays in care. Neurosurgeons can use surgery or endovascular embolization to treat this condition.

A Postoperative Complication of Takotsubo Syndrome in the Spinal Surgery: A Case Report.

We present the case of a 32-year-old woman with a diagnosis of lumbar root syndrome and spondylolisthesis, which is why she underwent surgery. Anterior discectomy and intersomatic box placement plus posterior fixation were performed with percutaneous transpedicular screws in L5-S1. At 24 hours of the procedure, the patient presents sustained hypotension, adding sudden and intense chest pain with neck irradiation, dyspnea, and diaphoresis, as well as electrocardiographic abnormalities and elevation of cardiac enzymes suggestive of an acute coronary syndrome, subsequently evidence of basal hypokinesis in the echocardiogram. After providing hemodynamic support and analgesic management, the symptoms were resolved, and the electrocardiogram (ECG) and cardiac enzymes were normalized, allowing an adequate postoperative evolution.

Resection of Thoracic Plasmacytoma and Corpectomy Through the Anterolateral Thoracic Approach: A Case Report.

This case report details the case of a 57-year-old male who initially manifested low back pain radiating from the lumbar region to the left leg. Progressive symptoms included paresthesia on the plantar surfaces of both feet and gait instability attributed to weakness in the pelvic limbs. Computed tomography imaging revealed osteolytic lesions in the T9, T10, and T11 vertebral bodies, resulting in compression of the spinal cord. Subsequent contrast-enhanced magnetic resonance imaging validated these findings, confirming the presence of an extradural tumor. In accordance with the Spinal Instability Neoplastic Score (SINS), the case was categorized as indicative of potential spinal instability. Consequently, a surgical intervention was performed to excise the lesion. Thus, the role of SINS played a pivotal role in guiding the decision-making process for the chosen treatment modality.

Characteristics and Long-Term Outcome of Cerebellar Strokes in a Single Health Care Facility in Mexico.

Objective The purpose of this study was to analyze and discuss the clinical characteristics, long-term outcome, and prognostic factors of cerebellar strokes treated in a single health care facility in Mexico. Methods We retrospectively reviewed the medical records of adult patients admitted to our hospital with diagnosis of cerebellar ischemic and hemorrhagic stroke between 2018 and 2020. Baseline data included sociodemographic and radiological variables, treatment (surgical versus conservative), and Glasgow Coma Scale on arrival (GCSOA). The final neurological outcome was evaluated with the Glasgow Outcome Scale (GOS) six months after hospital discharge. Results Ten patients (seven male and three female) with a mean age of 57.9 ± 9.3 years were included, six with cerebellar ischemic infarction and four with cerebellar hemorrhage. Out of the 10 patients, four underwent surgery (suboccipital decompressive craniectomy {SDC} ± ventriculostomy). The outcome was favorable in four cases (40%) and unfavorable in six (60%). Patients who underwent surgical treatment fared worse with all four cases associating poor outcome. The comparison between good and poor outcome groups showed significant differences in the presence of obstructive hydrocephalus (one versus six, p = 0.05) and poorer GCSOA (6.16 ± 1.72 versus 12.5 ± 3.6, p = 0.05), associating poorer outcome. Conclusion There is still controversy regarding the appropriate management of cerebellar strokes. The presence of obstructive hydrocephalus and poorer GCSOA are associated to worse outcomes.

Magnetic resonance imaging finding of coexistence of bilateral paraclinoid aneurysms in a patient with a nonfunctioning macroadenoma, simultaneous resection, and clipping: illustrative case.

BACKGROUND: Unruptured incidental intracranial aneurysm can coexist with pituitary adenoma, however, the occurrence is extremely rare. Timely diagnosis of asymptomatic intracranial aneurysms with pituitary adenoma may lead to planning a tailored surgical strategy to deal with both pathologies simultaneously. A case of a patient who underwent transcranial resection of a pituitary adenoma with clipping of two mirror aneurysms is reported. OBSERVATIONS: A 55-year-old female presented with deterioration of visual acuity that progressed over 1 year, as well as presence of right eyelid ptosis. Magnetic resonance imaging of the head showed the presence of an intrasellar pituitary macroadenoma. Bilateral paraclinoid aneurysms were documented to be in contact with the pituitary tumor. The patient underwent surgery with simultaneous aneurysm clipping and tumor resection through a standard pterional approach with intradural clinoidectomy. The aneurysms were successfully clipped after the tumoral debulking. After clipping, the pseudocapsule was fully resected. LESSONS: Various treatment options are available. Although endovascular securing of the aneurysms prior to the tumor resection would be ideal, in cases in which this resource is not readily available at all times, the surgeon must be prepared to solve pathologies with an elevated level of complexity.

Teratoma With Malignant Ectomesenchymoma in the Pineal Region: A Case Report.

Tumors involving the pineal gland include germinomas, non-germinomatous, and parenchymal tumors. Sometimes these tumors can be differentiated into rhabdomyosarcoma, which is an aggressive and rapidly recurring sarcoma but is a rare event. We present the case of a 23-year-old male, with an eight-year-long history of a non-treated brain tumor compatible with a teratoma. Chemotherapy and radiotherapy were offered, and two years later, malignant transformation to astrocytoma, rhabdomyosarcoma, neural cell carcinoma, ganglioglioma, and low-grade chondrosarcoma was noted. Immunohistochemistry was valuable in differentiating these entities that confirmed the diagnosis. Malignant transformations may be secondary to the normal transformation of multipotent embryonic cells into more developed tissues after radiotherapy of teratoma and malignant ectomesenchymoma transformation.

Carboplatin Plus Vincristine as an Alternative Chemotherapeutic Scheme in Patients With Glioblastoma.

Background and objective Alternative chemotherapy regimens, including cisplatin, carmustine, or other agents, have been shown to be effective; however, the use of carboplatin plus vincristine (C/V) has not been studied before. In this study, we aimed to determine the survival rates in patients treated with C/V, by comparing our findings with treatments based on temozolomide (TMZ), and to explore a possible relationship with the methylation status of the methylguanine methyltransferase (MGMT) promoter in patients with glioblastoma (GB). Methods A retrospective cohort study was conducted involving 45 surgically treated patients diagnosed with GB. Fresh tissue samples were examined by the DNA bisulfite conversion method to determine methylation status. After surgery, different chemotherapy regimens were employed as adjuvants. Follow-up of participants was performed as outpatients at three-month intervals to determine overall survival (OS), by comparing the use of TMZ versus C/V. Results MGMT promoter methylation status could only be determined in 35 samples; 20 patients received adjuvant chemotherapy, of which 14 were treated with C/V and six with TMZ-based schemes. The median OS (mOS) was eight months (range: 1-24 months). OS was 57.25% at six months, 48.7% at 12 months, and 28.5% at 24 months. In the TMZ group, an OS of 83% was observed at 24 months. In the C/V group, OS was 71.4% at six months, 57.1% at 12 months, and 35.7% at 24 months. Patients who did not receive adjuvant chemotherapy treatment had the lowest survival rates with an OS of 39.9% at six months, 26.6% at 12 months, and 19.9% ​​at 24 months. Conclusions Based on our findings, C/V offers an accessible and effective alternative treatment when the TMZ-based scheme is not accessible, providing higher rates of OS compared to patients without chemotherapy management. The methylation status of the MGMT promoter is a significant prognostic factor, resulting in higher survival rates among patients when it is methylated.

Surgical Approach and Further Management of Intracranial Hemangioendothelioma With Double Location: A Case Report.

Hemangioendotheliomas are highly vascularized lesions, and their intracranial presentation is extremely rare. We present the case of a 65-year-old female patient who was evaluated for cranial deformity, headache, and left hemiplegia. Two bone lesions that were destroying and expanding the bone diploe with intracranial extension were identified in the fronto-temporal and parietal regions. Both lesions were multilobed and showed heterogeneous behavior. Mixed hemangioendotheliomas were identified after the successful resection of both tumors in two separate surgical procedures. The prognosis of this type of tumor with an intracranial location is not well-defined because there are too few reported cases.

Bilateral Proximal Forearm Transplantation: Case Report at 7 Years.

BACKGROUND: Although return of function has been reported in patients undergoing proximal forearm transplantations (PFTs), reports of long-term function are limited. In this study, we evaluated the clinical progress and function 7 years postoperatively in a patient who underwent bilateral PFT. CASE PRESENTATION: A 58-year-old man underwent bilateral PFT in May 2012. Transplantation involved all of the flexor and extensor muscles of the forearm. Neurorrhaphies of the median, ulnar, and radial nerves were epineural and 7 cm proximal to the elbow. Immunosuppressive maintenance medications during the first 3 years postoperatively were tacrolimus, mycophenolate, and steroids, and later, tacrolimus, sirolimus, and steroids. Forearm function was evaluated annually using the Disabilities of the Arm, Shoulder, and Hand; Carroll; Hand Transplantation Score System; Short Form-36; and Kapandji scales. We also evaluated his grip and pinch force. RESULTS: Postoperatively, the patient developed hypertriglyceridemia and systemic hypertension. He experienced 6 acute rejections, and none were resistant to steroids. Motor function findings in his right/left hand were: grip strength: 10/13 kg; key pinch: 3/3 kg; Kapandji score: 6/9 of 10; Carroll score: 66/80; Hand Transplantation Score System score: 90/94. His preoperative Disabilities of the Arm, Shoulder, and Hand score was 50 versus 18, postoperatively; his Short Form-36 score was 90. This function improved in relation with the function reported in the second year. CONCLUSIONS: Seven years following PFT, the patient gained limb strength with a functional elbow and wrist, although with diminished digital dexterity and sensation. Based on data presented by other programs and our own experience, PFT is indicated for select patients.

Soft tissue angiosarcoma of the breast in a patient with Klippel-Trenaunay-Weber syndrome.

Angiosarcomas are neoplasms of blood or lymphatic vessels with aggressive behavior. We report the coexistence of this malignancy within soft tissue of the breast in a 49-year-old woman who was diagnosed with Klippel-Trenaunay-Weber syndrome (KTW-S) during childhood. The patient has no previous history of radiation therapy on the chest and does not have any known risk factor for developing angiosarcoma, except for her congenital disease. To the best of our knowledge, the association between soft tissue angiosarcoma of the breast and KTW-S has never been previously reported.

Los angiosarcomas son neoplasias de los vasos sanguíneos y linfáticos con comportamiento agresivo. Reportamos la coexistencia de esta malignidad dentro del tejido blando de la mama en una mujer de 49 años que fue diagnosticada de síndrome de Klippel-Trenaunay-Weber desde la infancia. La paciente no tiene antecedentes de radioterapia en el tórax ni factores de riesgo conocidos para desarrollar angiosarcoma, a excepción de su enfermedad congénita. A nuestro saber, la asociación entre el angiosarcoma del tejido blando de la mama y el síndrome de Klippel-Trenaunay-Weber no había sido reportada previamente.